1. Risk Factors For Permanent Hearing Loss Betty Vohr, M.D. Medical Director Rhode Island Hearing Assessment Program Professor of Pediatrics Brown Medical School

2. Causes of Permanent Hearing Loss in 100 Infants 50%Environmental50 50%Genetic 30% syndromes (>300)30 20% >75 genes ident20 ½ are GJB2 – Connexin 26

3. Genetic Causes Single geneConnexin 26 Gene + environmentMitochondrial + ototoxic Gene + geneGene + other gene

4. Dilemma #1: Two Sets of Risk Factors Neonatal Risk Factors Late onset Risk Factors

5. JCIH Neonatal Risk Indicators for neonates <28 d where there is no universal screening Illness with admission to NICU > 48 hours Stigmata assoc with SNHL or Conductive HL Family hx of permanent childhood SNHL Craniofacial anomalies In-utero infections ie CMV, herpes, toxoplasmosis or rubella

6. Risk Indicators for late onset HL Caregiver concern re: hearing, speech, language Family hx of permanent childhood HL Stigmata associated with SNHL or Conduct. HL Postnatal infections: ie meningitis In-utero infections ie CMV, herpes, toxoplasmosis or rubella Hyperbilirubinemia req Exchange, PPHN, ECMO Syndromes assoc with SNHL Neurodegenerative disorders Head Trauma & persistent OM with effusion

7. Checklist of speech –language milestones Birth to 3 months Startles to loud noise Awakens Blinks or widens eyes in response to sound 3-4 months Quiets to mother’s voice Stops playing, listens to new sounds Looks for source of sounds not in sight 6-9 months Enjoys musical toys Coos and gurgles with inflection Says “mama”

8. Checklist of speech –language milestones 12-15 months responds to his or her name and “no” Follows simple requests Uses expressive vocabulary: 3-5 words Imitates sounds 18-24 months knows body parts 20-50 words, 2 word phrases 50% of speech intelligible to strangers 36 months approximately 500 words, 4-5 word sentences 80% intelligible to strangers: understands some verbs

9. JCIH Recommendation All children who pass the newborn screen but have risk indicators for auditory disorders or speech and language delay should receive ongoing audiologic and medical monitoring for HL and language development for the first 3 years of life.

10. Dilemma #2: 2 sets of babies The NICU graduate- Considered high risk for many ND sequelae. Many have routine longitudinal surveillance for 1-3 years and are less likely to fall through the cracks. The well baby nursery baby with a risk factor. Monitoring considered less important by family and primary provider and therefore more likely to be lost to FU.

11. Questions ? How is tracking of risk factors accomplished ? Is it cost effective to track all children with a risk factor ? Can it be accomplished on large populations ?

12. Longitudinal Assessment of Infants who pass but have a risk factor Whose responsibility is it ? The State EHDI system ? The Medical Home ? The Family ?

13. State EHDI Systems Tracking must be centralized There needs to be a centralized data management system. We need to have quality indicators for tracking infants with risk factors.

14. Data Management System Requirements Electronically store core demographic and hearing screen & assessment data elements Modifiable data model- protocols change Multi-user ability to access & retrieve data Protect and ensure security of confidential health care information Tracking and follow up capability

15. Benchmarking a Hearing Screen Program Infants screened 99% 1st stage (TEOAE/AABR) fail rate<4% Rescreen return rate>90% Diagnostic procedures on refers>90% Referral age for intervention services<6m Number referred to EI 100% Follow-up of Infants with Risk Factors est 40%

16. Tracking for Risk Factors in Rhode Island NICU – thorough chart review completed to identify risk factors Well Baby Nursery – Risk factors identified through notations in the child’s Kardex entry and through nurse’s report

17. VRA Referral Process Primarily Risk Factors After newborn screen Letter to parent and PCP Audiology Network list At age 5 months Reminder letter to parents At age 6 months Second letter to parents

18. Diagnostic Protocol -six months corrected age Visual Reinforcement Audiometry – insert earphones – hidden, eye-level reinforcers – 2-tester paradigm TEOAE or DPOAE Tympanometry/Acoustic Reflexes

19. Reporting of Dx Results Results requested for all RIHAP referrals To ensure all children receive follow-up Reporting is NOT mandatory Percentage Received VRA – 40%

20. Reporting of Dx Results Reports reviewed and coded by audiologist when received Referral to Early Intervention if appropriate RIHAP as “safety net” for EI referrals Results entered into RITRACK by Data Staff

21. RIHAP Referrals 2002 13,568 initial screens 232 (1.7%) did not pass the screen prior to discharge 379 (2.8%) passed and were referred on for monitoring due to risk factors

22. Determinants of successful Follow-up Medical Home awareness of risk factors, speech and language milestones, and the importance of follow-up. Family awareness of risk factors, speech and language milestones and importance of follow-up. A reliable tracking system

23. Unresolved issues What is the risk of late onset hearing loss for the current individual risk factors ? Are there adequate resources for tracking all children with risk factors ?